Liver Transplantation for Zellweger Syndrome
© 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation..
Zellweger syndrome or cerebrohepatorenal syndrome is a rare, multisystem disorder occurring due to defect in metabolic pathway within the peroxisomes. Cirrhosis with portal hypertension is an important presentation of these patients. Given its progressive, multisystem nature, the role of liver transplantation (LT) in Zellweger syndrome remains undefined and controversial. An 11-y-old boy diagnosed with Zellweger syndrome presented to the authors with decompensated cirrhosis along with bilateral proptosis. After a meticulous evaluation, he was offered an ABO incompatible liver transplantation with his mother being the donor. He had an uneventful post operative period. After a follow up of 24 mo, he has normal graft function, normal cognition along with resolution of proptosis. Therefore, in a group of carefully selected patients with Zellweger syndrome, a liver transplantation can be offered successfully with an excellent prognosis.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:91 |
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| Enthalten in: |
Indian journal of pediatrics - 91(2024), 5 vom: 01. Apr., Seite 507-509 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Menon, Jagadeesh [VerfasserIn] |
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| Links: |
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| Themen: |
Cirrhosis |
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| Anmerkungen: |
Date Completed 19.04.2024 Date Revised 19.04.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s12098-023-04937-7 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
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| 520 | |a © 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation. | ||
| 520 | |a Zellweger syndrome or cerebrohepatorenal syndrome is a rare, multisystem disorder occurring due to defect in metabolic pathway within the peroxisomes. Cirrhosis with portal hypertension is an important presentation of these patients. Given its progressive, multisystem nature, the role of liver transplantation (LT) in Zellweger syndrome remains undefined and controversial. An 11-y-old boy diagnosed with Zellweger syndrome presented to the authors with decompensated cirrhosis along with bilateral proptosis. After a meticulous evaluation, he was offered an ABO incompatible liver transplantation with his mother being the donor. He had an uneventful post operative period. After a follow up of 24 mo, he has normal graft function, normal cognition along with resolution of proptosis. Therefore, in a group of carefully selected patients with Zellweger syndrome, a liver transplantation can be offered successfully with an excellent prognosis | ||
| 650 | 4 | |a Journal Article | |
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| 650 | 4 | |a Liver transplantation | |
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| 650 | 4 | |a Zellweger syndrome | |
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| 700 | 1 | |a Rammohan, Ashwin |e verfasserin |4 aut | |
| 700 | 1 | |a Rela, Mohamed |e verfasserin |4 aut | |
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